Among infectious factors, varicella-zoster virus (VZV) is a leading cause of central nervous system vasculopathy and stroke in childhood. Not only have viral markers been detected in the cerebrospinal fluid of affected patients, but also direct evidence of viral particles in the wall of cerebral arteries has been demonstrated in rare pathological specimens. This certainly reflects a localized infectious process likely associated with variable indirect inflammatory responses. Yet the usefulness in this setting of a lumbar puncture as well as of subsequent targeted antiviral and/or anti-inflammatory therapies is uncertain. Indeed, in the majority of cases, the so-called post-varicella angiopathy has a monophasic evolution with spontaneous resolution or stabilization, explaining diverging diagnostic and treatment approaches. In this paper, we have addressed this problematic area by reviewing 26 published cases from the year 2000 and three unpublished cases. Post-varicella stroke is typically associated with angiopathy most often involving the initial portion of the middle cerebral artery, causing a basal ganglia stroke. It tends to occur in young immunocompetent children. Thrombophilia work-up is in general negative. Lumbar puncture was performed in 17 out of 29 cases. Viral markers were examined in 14 cases, but were positive in only eight cases. Antiviral therapy was administrated in 11 children. In this small retrospective study, the treated children's vasculopathy did not progress more favorably nor was there a better outcome compared with untreated subjects.